RCVS – Reversible Cerebral Vasoconstriction
Syndrome
• Segmental cerebral vasoconstriction usually associated with acute onset of severe (thunderclap) headache, reversible (within 3 months)
• Other names:
o Call-Fleming Syndrome
o Thunderclap headache with reversible vasospasm
o Benign angiopathy of the CNS (BACNS)
o Migrainous vasospasm or angiitis
o Drug induced cerebral vasculopathy
o The name RCVS was proposed in 2007
• Can result in PRES however is not synonymous (see PRES topic)
• Age 10-76yr in literature
• Peak age 42
• Female >Male
• Incidence ?? no good data
• Risk factors:
o ?History of migraine
• Primary causes
o “Primary thunderclap headache”
o Exertional headache, headache associated with cough/bathing/sexual activity
o N.B. although these primary headaches can be associated with vasospasm often they do not appear to be and conceivably may have a variety of causes.
• Secondary causes
o Pregnancy/Postpartum
- Either ‘idiopathic’ or associated with eclampsia or vasoactive drugs given during pregnancy
o Vasoactive substances
- Illicit drugs
• Cannabis, cocaine, ecstasy, amphetamines, LSD
- Ergots
• Ergotamine, bromocriptine, methergine
- Sympathomimetics
• Ephedrine, pseudoephedrine, diet pills
- Serotonergic drugs
• SSRI, SNRI, triptans
- Immunosuppressants
• Tacrolimus, cyclophosphamide, IFN-a
- Other
• Indomethacin, OCP, nicotine patches
• Ginseng
• Alcohol – binge
o Catecholamine secreting tumours
- Phaeochromocytoma
- Bronchial carcinoid tumour
- Glomus tumour
o Arterial/Vascular disorders
- Dissection
- CEA
- PRES
- ?CVST, ?Aneurysm
o Blood products
- EPO, IVIG, massive transfusion
o Intracranial disorders
- ICH, trauma, intracranial hypotension, neurosurgery
o Misc
- Hypercalcaemia, SLE, Autonomic dysreflexia, phenytoin intoxication, porphyria
• Thunderclap headache (Thunderclap Headache)
o Initial symptom in >80%
o Often can be recurrent (2-10 headaches, mean of 4) over 1- 4 weeks
o More often posterior onset
o Last minutes to days (usually 1-3 hours) – this is shorter than SAH
o A moderate, ‘background’ headache can persist between attacks
• Seizures (1%-17%)
o Recurrent seizures are rare
• Focal neurological deficits (8-43%)
o Most often visual
• Hypertension - 1/3 patients
o ?pain vs underlying aetiology
• Major differential diagnosis is SAH for clinical presentation and vasculitis for radiological findings
o CT +/- LP +/- CTA to exclude SAH
o MRI brain – to look for alternative causes and complications of RCVS
o If there is reason to suspect vasculitis investigation for this – including biopsy should be considered.
• Vascular imaging
o The vasospasm can be delayed – up to 3 weeks (mean 16 days) to reach maximal, initial scanning can be negative
o Segmental narrowing, ‘string of beads’
o Modality depends on local access:
- Catheter angiography – gold standard
- Non-invasive
• Sensitivity of non-invasive imaging ~70%
• MRA/CTA/USS
• Transcranial doppler may be useful for monitoring progress of disease
• Response to intravascular nimodipine has been proposed but not proven
• Lumbar puncture
o To exclude other causes
o White cell count
- Elevation up to 35 has been reported
- >10 in 3-8%
- 5-10 in up to17%
o Protein >600mg/L in up to16%
o Has been suggested to repeat LP in a few weeks if WCC >10 or Protein >800mg/L
• Bloods
o Could consider vasculitis screen
o Consider screen for phaeochromocytoma
o Consider Drug screen
• Proposed diagnostic criteria (Calabrese et al. 2007):
o Documentation of multifocal segmental cerebral artery vasospasm
o No evidence of aneurysmal SAH
o Normal or near normal CSF
o Severe, acute headaches (with or without other focal neurological signs)
o Reversibility within 12 weeks (or significant improvement ?). If death occurs within this time-frame autopsy evidence ruling out vasculitis or other causes.
• SAH – has to be excluded first in all cases of thunderclap headache
• Cerebral vasculitis
o More insidious onset
o Progressive symptoms
o MRI shows infarcts on initial scan in >90% of cases
o Inflammatory CSF in >95%
o Vasoconstriction often not visualised
• A number of complications have been attributed to RCVS (although it could not be ruled out that some are causative rather than complications)
|
TIA |
16% |
|
|
PRES |
9-14% |
Usually with onset,
often associated with stroke |
|
Cerebral oedema |
38% |
|
|
ICH |
up to 20% |
Often occur with the thunderclap headache |
|
Cortical/convexity SAH |
22 to 34% |
Half seen within first week |
|
SDH |
2% |
|
|
Stroke |
6-39% |
Watershed, often between PCA/MCA, usually occur later (mean 9-10 days, reported up to 17 days) |
• Permanent neurological deficits - 3-9%
• Stroke deficits are usually relatively small
• Rarely case reports of mortality (<1%)
• Recurrence rate unknown
o In one study (from Taiwan of 168pts) 10.7% of patients had recurrent thunderclap headache and 5.4% had proven recurrence at a mean of 41 months post initial episode. None developed complications. (Neurology 2015)
• Pain relief
• Identification and elimination of exacerbating factors
• Manage complications:
o E.g. Seizures
• Blood pressure
o ?Manage as per acute stroke – i.e. do not aim for rapid reduction
• Treatment of vasospasm
o Nimodipine
- 1-2mg/hr IV adapted to blood pressure then oral 30-60mg Q4H for 4-8 weeks
- Reduced number and intensity of headaches
- Did not change time course of vasospasm
- Complications (TIA/Stroke/ICH) have occurred despite treatment
- One paper suggests early treatment improves resolution – with no difference in complications (Effect of Nimodipine Treatment on the Clinical Course of Reversible Cerebral Vasoconstriction Syndrome. Front Neurol. 2019;10:644.)
o Verapamil (case reports of ‘effectiveness’)
o Magnesium sulphate
• Steroids
o No proven benefit – concern about worsening.
• Intraarterial vasodilators
o Case reports with ‘debatable success